Congenital generalized fibromatosis with visceral involvement. A case report

Abstract

A case of congenital generalized fibromatosis in a black male infant with involvement of lungs, subcutaneous tissue, pancreas, adrenal, lymph nodes, and bone is described. Fifteen prior cases with pulmonary involvement are reviewed, emphasizing the poor prognosis associated with pulmonary lesions. This case demonstrated, in addition to previously described histologic features, broad bands of fibroblastic proliferation in the interlobular septa producing retraction of the visceral pleura. Possible etiologic factors are discussed, with suggestions for additional studies in future cases.Cancer 45:954-960, 1980. ONGENITAL GENERALIZED FIBROMATOSIS (CGF)C is a rare entity,23 involving the formation of nodules or masses of well differentiated fibrous tissue in the viscera, musculoskeletal system, and subcutaneous tissue. In fatal cases, death occurs in the first few months of life.1,15*19 In those infants who survive, spontaneous regression of the masses has been described.1s*20,21,2s Pulmonary involvement in CGF seems to carry a particularly grave prognosis. Fifteen cases of fibromatosis involving the lungs have been ~e S c~~~e~, 2 ,~,~, R , 1 0 ' " 4 . ' " . ' " . 2 2 -2 4 , 2~, Z 7 and all but 2 patients died.x*19 We report a case of CGF with pulmonary involvement in a black infant. Case ReportA 4-month-old black boy was seen because of tachypnea and a one-week history of fever and anorexia. The infant, who weighed 1500 g at birth, was born to a primigravida woman after 35 weeks of gestation. Two purplish-red masses on the posterior aspect of the boy's head and the left side of the neck noted at birth were thought to be hemangiomas.Physical examination revealed that the infant was afebrile with a respiratory rate of 100 and pulse of 140imin. His weight, 4275 g, was below the 3rd percentile. The occipital mass had completely regressed, and the left cervical mass measured 5 x 5 x 1 cm. Right lower lobe rales, a Grade IIiVI systolic ejection murmur, and an S, gallop were noted on auscultation. Hepato-splenomegaly was present. Electrocardiographic studies demonstrated right axis deviation and right ventricular hypertrophy. The chest x-ray showed a n interstitial infiltrative process. The hemoglobin was 10.6 gidl, hematocrit 3296, white blood count 69001pL1, with 41% segmented neutrophils, 40% lymphocytes, 5% band neutrophils, and 14% monocytes. An open lung biopsy from the right upper lobe showed minimal interstitial mononuclear inflammation, mild intraalveolar edema, and Type I1 pneumonocyte hyperplasia. On the seventh postoperative day, the infant suffered a cardiac arrest and could not be resuscitated. Autopsy FindingsThe striking findings were in the respiratory and cardiovascular systems. The surface of the lungs was grossly nodular; the lungs were firm and noncrepitant. Some of the interlobular septa were prominently thickened by gray-white tissue, and small white spherical nodules, 1 mm or less in diameter, were occasionally noted in the parenchyma of the lung (Fig. 1). The heart was enlarged, with right ventricu...

Giant pancreatic solid cystic desmoid tumor with two ectopic adrenal tissues

“…Symptoms depend on the location and occur with local invasion to adjacent tissues (7). The patients with intraabdominal DT generally have no symptoms and are encountered incidentally during laparotomy.…”

Robotic-Assisted Distal Pancreatectomy for a Pancreatic Desmoid Tumor: A Case Report and Review of the Literature

“…Our case is the twenty-first case of a pancreatic DT reported in the English literature; twenty previous cases have been identified and are summarized in Table 1 [7,9,11,16,[34][35][36][37][38][39][40][41][42][43][44][45]. DTs occurring in the pancreas, unlike other intra-abdominal DTs, are most commonly sporadic with only one reported pancreatic tail solid/cystic DT reported to be associated with FAP.40 Tumors were equally distributed between genders (12 males and 9 females).…”

Development of renal and iliac aneurysms in a child with generalized infantile myofibromatosis

“…However, vascular damage of large blood vessels is less frequent. Lesions of the pulmonary vein and other large veins have been described occasionally [7,8]. Wright et al have reported a patient with multifocal IM who subsequently developed several aneurismal dilations of the arterial system including the thoracic and abdominal aorta, carotid, iliac, and lower limb vessels.…”